PC1202: A Case of Pediatric Sarcoidosis Presenting with Bilateral Parotid Gland Swelling

Introduction: Pediatric sarcoidosis is a rare granulomatous inflammatory condition. It varies in presentation but classically demonstrates noncaseating granulomas in tissues and organ systems. Blau syndrome is a form of sarcoidosis that can present in children with a triad of polyarthritis, uveitis, and rash. However, children can also present with the adult form of sarcoidosis characterized by pulmonary and lymph node involvement.

Case Description: A 9-year-old previously healthy boy presented with 10 days of low-grade fever, cough, tender cervical lymphadenopathy, and bilateral parotid gland swelling. Initial labs showed neutrophil predominant leukocytosis, normocytic anemia, normal platelet count, elevated CRP, and elevated ESR. A CT scan of the neck showed diffuse bilateral cervical lymph node prominence without central necrosis or abscess. His symptoms were initially thought to be due to bacterial parotitis however he did not improve with antibiotics. He was fully immunized against the Mumps virus. A cervical lymph node biopsy was done which demonstrated numerous non-caseating granulomas with multinucleated giant cells (Figure 1). Histochemical stains for microorganisms were negative and flow cytometry provided no evidence of lymphoma. A CT of his thorax showed ground glass opacities, interstitial thickening, and hilar adenopathy (Figure 2). An extensive infectious workup was negative including testing for EBV, CMV, toxoplasmosis, Bartonella, HIV, histoplasmosis, tuberculosis, and viral respiratory panel. Blood and fungal cultures showed no growth. Additional labs revealed a negative ANA, negative Sjogren’s antibodies (anti-SSA and anti-SSB), negative ANCA, normal angiotensin-converting enzyme (ACE), elevated lysozyme, normal creatinine, and normal urinalysis. Based on the combination of bilateral parotitis, non-caseating granulomas, ground glass opacities, and hilar adenopathy, a diagnosis of sarcoidosis was made. He was started on daily prednisone in addition to weekly subcutaneous Methotrexate. Screening for other organ involvement such as uveitis and cardiac involvement was negative.

Discussion: Sarcoidosis should be included in the differential diagnosis for a patient presenting with bilateral parotitis. There are no laboratory tests to diagnose sarcoidosis and elevation of the serum ACE level is not always present. The diagnosis can be supported by the characteristic pathology of noncaseating granulomas in addition to pulmonary and lymph node involvement. Other organ systems can be involved, and patients should be screened for uveitis, renal, and cardiac involvement. Noncaseating granulomas can also be seen in the setting of certain infections or malignancy, and these should be ruled out prior to treatment with immunosuppressive medications. Treatment of sarcoidosis involves corticosteroids and often a steroid sparing medication such as methotrexate, a TNF antagonist, azathioprine, or mycophenolate. Some patients may experience spontaneous resolution of their symptoms and may not require treatment.

Conclusion: Although rare, sarcoidosis should be considered in the differential diagnosis of bilateral parotitis and in cases where biopsies demonstrate non-caseating granulomas.